MISSED IMMUNO

Question 1

absence of thymic shadow

Answer 1

Think Digeorge or SCID..

with SCID –> adenosine deaminase ADA deficiency or mutations in gamma chain shared by interleukin receptors.

Question 2

No thymic shadow, low lymphocytic count, flow cytometry has Low T and B cells, low levels of all immunoglobulins

Answer 2

SCID.
X linked
Autosomal recessive
TX: infections with antibiotics. Bone marrow transplant

Question 3

largest antibody, binds and activates complement, formed early in immune response

Answer 3

IgM

Question 4

only immunoglobulin that can cross the placenta, involved in opsonization of bacteria, neutralizes viruses and bacterial toxins, binds and activates complement, delayed formation during the course of infection

Answer 4

IgG

Question 5

Binds to mast cells to mediate histamine release, binds to basophils, involved in defense against parasites by stimulating eosinophils

Answer 5

IgE

Question 6

Found on the surface of mature B lymphocytes and blood serum

Answer 6

IgD

Question 7

Found on mucosal surfaces and in bodily fluids (saliva, mucus), prevents binding of pathogens to mucous membranes

Answer 7

IgA

Question 8

Defective IL-2R gamma chain receptor or ADA deficiency, or recombination activating gene (RAG) mutation leads to

Answer 8

SCID

Question 9

decreased IgA, IgG, IgE, increased AFP and decreased lymphocytes

Answer 9

Ataxia Telengiectasia

Question 10

lysosomal trafficking regulator (LYST) gene mutation, defective microtubule polymerization in phagosome lysosome fusion

Answer 10

Chediak Higashi Syndrome

Question 11

Defect of NADPH oxidase –> decrease reactive oxygen species and decreases respiratory burst in neutrophils. Abnormal dihydrohodamine test

Answer 11

Chornic Granulomatous disease

Question 12

B cells unable to differentiate into plasma cells, decreased plasma cells, decrease Ig of all classess. Recurrent sinopulmonary infections, increased risk of lymphoma and autoimmune disorders

Answer 12

Common variable immunodeficeincy

Question 13

Decreased T cells, PTH, Ca2+

Answer 13

DiGeorge

Question 14

Signal transducer and activator of transcription 3 (Stat3 gene) mutation –> TH17 cells impaired neutrophil recruitment to sites of infection. Retained primary teeth, coarse facies. Increased IgE, decreased IGN-gamma,increased eosinophils

Answer 14

Hyper IgE

Question 15

CD40 Ligand Deficiency on Th cells –> defective B cells class switching. Everything decreased but increase IgM

Answer 15

Hyper IgM

Question 16

Absence of LFA integrin protein on phagocytes. Leukocytes unable to migrate to tissues during infection or inflammation. Dysfunctional neutrophils –> absent pus –> impaired wound healing. Delayed separation of umbilical cord. Increase in neutrophils in blood

Answer 16

Leukocyte Adhesion Deficiency Type 1

Question 17

Mutation in WAS gene, leukocytes and platelets unable to reorganize actin cytoskeleton –> defective antigen. Normal or decreased IgG and IgM. increased IgE and IgA, thrombocytopenia with small platelets

Answer 17

Wisckott-Aldrich syndrome

Question 18

BTK gene defect –> no B cell maturation. Recurrent bacterial and enteroviral infections

Answer 18

X linked (bruton) agammaglobulinemia

Question 19

Hypersensitivity of anaphylaxis and asthma?

Answer 19

Type 1

Question 20

Hypersensetivity of Graves, drug induced hemolytic anemia, Myasthenia gravis?

Answer 20

Type 2

Question 21

Hypersensitivity of poststreptococcal glomerulonephritis

Answer 21

Type 3

Question 22

Serum sickness hypersensitivity

Answer 22

TYpe 3

Question 23

Poison ivy dermatitis, nickel derm.. hypersensitivity

Answer 23

4

Question 24

immune complex mediated hypersensitivity reaction that occurs when the body forms antibodies against foreign proteins (antigens from drugs, non human antibodies).

Onset 1-2 weeks after exposure to offending antigen or drug.

Common triggers: Non human proteins (historically from serum used for antitoxins).
Drugs: penicillin, sulfoniamides, allopurinol, phenytoin.

Fever, urticarial rash, arthralgia, lymphadenopathy, proteinuria, viral illness or vasculitis

Answer 24

Serum sickness

Question 25

A 9-year-old boy is brought to the clinic with a rash, joint pain, and fever. He was treated with amoxicillin-clavulanate for streptococcal pharyngitis 10 days ago. Today, he complains of swollen knees and a painful, itchy rash on his trunk and arms. Physical exam reveals urticarial lesions, low-grade fever, and mild cervical lymphadenopathy. Urinalysis shows mild proteinuria. His serum complement levels are decreased. Which of the following best explains this patient’s condition?

Answer 25

Type III hypersensitivity reaction Serum sickness